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Dalmau, josep

Lidia Sabater, Jesús Planagumà, Josep Dalmau, Francesc Graus
BACKGROUND: Antibodies against IgLON5, a neuronal adhesion protein of unknown function, are markers of a novel neurological disorder termed anti-IgLON5 syndrome. The disorder shows a remarkable association with the HLA-DQB1*0501 and HLA-DRB1*1001 alleles, and postmortem studies demonstrate a novel neuronal tauopathy predominantly involving the hypothalamus and tegmentum of the brainstem. The role of IgLON5 antibodies in the pathogenesis of the disease is currently unknown. Here, we have determined the target epitopes of IgLON5 antibodies, the effects of the IgLON5 antibodies in rat hippocampal neurons, and the IgG subclass responsible for these effects...
2016: Journal of Neuroinflammation
Josep Dalmau
No abstract text is available yet for this article.
August 2016: Neurology® Neuroimmunology & Neuroinflammation
Ricardo Erazo, Jaime González, Consuelo Quintanilla, Claudia Devaud, Consuelo Gayoso, Ximena Toledo, Erna Rauch, Claudia Riffo, Carolina Alvarez, Marne Salazar, Daniela Salvo, Josep Dalmau, Orietta Carmona
INTRODUCTION: Subacute anti-NMDA receptor encephalitis was recognised in 2007 as a clinical entity, and was first described in young women with ovarian teratoma. The first paediatric series unrelated with tumours was reported in 2009. OBJECTIVE: To present the clinical features, treatment, and prognosis of 13 patients with anti-NMDA receptor encephalitis in Chile. PATIENTS AND METHOD: A description is presented of 13 children, 9 males, aged between 1 and 16 years, diagnosed between 2009 and 2016 in 7 hospitals...
July 28, 2016: Revista Chilena de Pediatría
Helena Ariño, Thais Armangué, Mar Petit-Pedrol, Lidia Sabater, Eugenia Martinez-Hernandez, Makoto Hara, Eric Lancaster, Albert Saiz, Josep Dalmau, Francesc Graus
OBJECTIVE: We investigated a series of patients with LGI1 antibody (Ab)-related cognitive deterioration to determine the clinical presentation, long-term outcome, and LGI1 Ab evolution. METHODS: We retrospectively analyzed the clinical information of 76 patients with LGI1 Ab-related cognitive deterioration. Presenting syndromes were classified as limbic encephalitis (LE), non-LE, or encephalopathy (normal MRI and no CSF pleocytosis). Frequency of relapses and clinical outcome were assessed in 48 patients with prolonged follow-up (median 39 months, range 18-200)...
August 23, 2016: Neurology
Jesús Planagumà, Holger Haselmann, Francesco Mannara, Mar Petit-Pedrol, Benedikt Grünewald, Esther Aguilar, Luise Röpke, Elena Martín-García, Maarten J Titulaer, Pablo Jercog, Francesc Graus, Rafael Maldonado, Christian Geis, Josep Dalmau
OBJECTIVE: To demonstrate that ephrin-B2 (the ligand of EphB2 receptor) antagonizes the pathogenic effects of patients' N-methyl-D-aspartate receptor (NMDAR) antibodies on memory and synaptic plasticity. METHODS: One hundred twenty-two C57BL/6J mice infused with cerebrospinal fluid (CSF) from patients with anti-NMDAR encephalitis or controls, with or without ephrin-B2, were investigated. CSF was infused through ventricular catheters connected to subcutaneous osmotic pumps over 14 days...
September 2016: Annals of Neurology
Estefanía Torres-Vega, María Durán-Moreno, Manuel Sánchez Del Pino, Yania Yáñez, Adela Cañete, Victoria Castel, Rogelio López-Cuevas, Juan Jesús Vílchez, Josep Dalmau, Francesc Graus, José Manuel García Verdugo, Luis Bataller
We aimed to identify new cell-membrane antigens implicated in opsoclonus-myoclonus with neuroblastoma. The sera of 3 out of 14 patients showed IgG electron-microscopy immunogold reactivity on SH-SY5Y neuroblastoma cells. Immunoprecipitation experiments using rat brain synaptosomes and SH-SY5Y cells led to the identification of: (1) thirty-one nuclear/cytoplasmic proteins (including antigens HuB, HuC); (2) seven neuronal membrane proteins, including the Shaw-potassium channel Kv3.3 (KCNC3), whose genetic disruption in mice causes ataxia and generalized muscle twitching...
August 15, 2016: Journal of Neuroimmunology
Agnes van Sonderen, Helena Ariño, Mar Petit-Pedrol, Frank Leypoldt, Peter Körtvélyessy, Klaus-Peter Wandinger, Eric Lancaster, Paul W Wirtz, Marco W J Schreurs, Peter A E Sillevis Smitt, Francesc Graus, Josep Dalmau, Maarten J Titulaer
OBJECTIVE: To report a large cohort of patients with antibodies against contactin-associated protein-like 2 (Caspr2) and provide the clinical spectrum of this disorder. METHODS: Serum and CSF samples were assessed at 2 neuroimmunology centers in Barcelona and Rotterdam. Patients were included if Caspr2 antibodies were confirmed with 2 independent techniques, including brain immunohistochemistry and cell-based assay. Clinical information was obtained by the authors or provided by treating physicians after patients' informed consent...
August 2, 2016: Neurology
Ellen Gelpi, Romana Höftberger, Francesc Graus, Helen Ling, Janice L Holton, Timothy Dawson, Mara Popovic, Janja Pretnar-Oblak, Birgit Högl, Erich Schmutzhard, Werner Poewe, Gerda Ricken, Joan Santamaria, Josep Dalmau, Herbert Budka, Tamas Revesz, Gabor G Kovacs
We recently reported a novel neurological syndrome characterized by a unique NREM and REM parasomnia with sleep apnea and stridor, accompanied by bulbar dysfunction and specific association with antibodies against the neuronal cell-adhesion protein IgLON5. All patients had the HLA-DRB1*1001 and HLA-DQB1*0501 alleles. Neuropathological findings in two patients revealed a novel tauopathy restricted to neurons and predominantly involving the hypothalamus and tegmentum of the brainstem. The aim of the current study is to describe the neuropathological features of the anti-IgLON5 syndrome and to provide diagnostic levels of certainty based on the presence of associated clinical and immunological data...
October 2016: Acta Neuropathologica
Josep Dalmau
No abstract text is available yet for this article.
June 2016: Neurology® Neuroimmunology & Neuroinflammation
Tanya J Williams, David R Benavides, Kelly-Ann Patrice, Josep O Dalmau, Alexandre Leon Ribeiro de Ávila, Dung T Le, Evan J Lipson, John C Probasco, Ellen M Mowry
IMPORTANCE: Paraneoplastic encephalitides usually precede a diagnosis of cancer and are often refractory to immunosuppressive therapy. Conversely, autoimmune encephalitides are reversible conditions that can occur in the presence or absence of cancer. OBJECTIVE: To report the induction of autoimmune encephalitis in 2 patients after treatment of metastatic cancer with a combination of the immune checkpoint inhibitors nivolumab and ipilimumab. DESIGN, SETTING, AND PARTICIPANTS: A retrospective case study was conducted of the clinical and management course of 2 patients with progressive, treatment-refractory metastatic cancer who were treated with a single dose each (concomitantly) of the immune checkpoint inhibitors nivolumab, 1 mg/kg, and ipilimumab, 3 mg/kg...
August 1, 2016: JAMA Neurology
Jessica A Panzer, Ronan Anand, Josep Dalmau, David R Lynch
No abstract text is available yet for this article.
June 3, 2016: Journal of Neuroimmunology
Henry Koiti Sato, Pedro André Kowacs, Josep Dalmau, Paulo Sergio Faro Santos
No abstract text is available yet for this article.
May 20, 2016: Arquivos de Neuro-psiquiatria
Nuria Gresa-Arribas, Jesús Planagumà, Mar Petit-Pedrol, Izumi Kawachi, Shinichi Katada, Carol A Glaser, Mateus M Simabukuro, Thaís Armangué, Eugenia Martínez-Hernández, Francesc Graus, Josep Dalmau
OBJECTIVE: To report a novel autoimmune encephalitis in which the antibodies target neurexin-3α, a cell adhesion molecule involved in the development and function of synapses. METHODS: Five patients with encephalitis and antibodies with a similar pattern of brain reactivity were selected. Antigen precipitation and determination of antibody effects on cultured rat embryonic neurons were performed with reported techniques. RESULTS: Immunoprecipitation and cell-based assays identified neurexin-3α as the autoantigen of patients' antibodies...
June 14, 2016: Neurology
Francesc Graus, Helena Ariño, Josep Dalmau
No abstract text is available yet for this article.
July 1, 2016: JAMA Neurology
Maria Sepúlveda, Thaís Armangue, Eugenia Martinez-Hernandez, Georgina Arrambide, Nuria Sola-Valls, Lidia Sabater, Nieves Téllez, Luciana Midaglia, Helena Ariño, Patrick Peschl, Markus Reindl, Alex Rovira, Xavier Montalban, Yolanda Blanco, Josep Dalmau, Francesc Graus, Albert Saiz
The aim of this study was to report the clinical spectrum associated with antibodies to myelin oligodendrocyte glycoprotein (MOG) in adult patients, and to assess whether phenotypic variants are dependent on recognition of rodent MOG epitopes. We retrospectively analyzed the features, course and outcome of 56 patients whose samples were investigated by brain tissue immunohistochemistry and cell-based assays using human and rodent MOG. The median age at symptom onset was 37 years (range 18-70); 35 patients (63 %) were female...
July 2016: Journal of Neurology
Joseph C Masdeu, Josep Dalmau, Karen F Berman
Since the early 1990s it has been postulated that hypofunction of N-methyl-d-aspartate (NMDA) receptors in brain networks supporting perception and cognition underlies schizophrenic psychosis. Recently, NMDA receptor hypofunction was described in patients with psychotic manifestations who exhibited autoantibodies binding the GluN1 subunit of the receptor, and who improved when the level of these antibodies was lowered by immunomodulation. In this disorder, NMDA receptor antibodies decrease the availability of NMDA receptors by internalizing them...
March 22, 2016: Trends in Neurosciences
Takahiro Iizuka, Juntaro Kaneko, Naomi Tominaga, Hidehiro Someko, Masaaki Nakamura, Daisuke Ishima, Eiji Kitamura, Ray Masuda, Eiichi Oguni, Toshiyuki Yanagisawa, Naomi Kanazawa, Josep Dalmau, Kazutoshi Nishiyama
IMPORTANCE: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disorder that occurs with IgG antibodies against the GluN1 subunit of NMDAR. Some patients develop reversible diffuse cerebral atrophy (DCA), but the long-term clinical significance of progressive brain and cerebellar atrophy is unknown. OBJECTIVE: To report the long-term clinical implications of DCA and cerebellar atrophy in anti-NMDAR encephalitis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective observational study and long-term imaging investigation was conducted in the Department of Neurology at Kitasato University...
June 1, 2016: JAMA Neurology
Josep Dalmau
No abstract text is available yet for this article.
April 2016: Neurology® Neuroimmunology & Neuroinflammation
Eugenia Martinez-Hernandez, Helena Ariño, Andrew McKeon, Takahiro Iizuka, Maarten J Titulaer, Mateus M Simabukuro, Eric Lancaster, Mar Petit-Pedrol, Jesús Planagumà, Yolanda Blanco, Robert J Harvey, Albert Saiz, Francesc Graus, Josep Dalmau
IMPORTANCE: Symptoms of stiff-person syndrome (SPS), stiff-limb syndrome (SLS), or progressive encephalomyelitis with rigidity, myoclonus, or other symptoms (SPS-plus) can occur with several autoantibodies, but the relative frequency of each antibody, syndrome specificity, and prognostic implications are unclear. OBJECTIVE: To report the clinical and immunologic findings of a large cohort of patients with stiff-person spectrum disorder (SPSD), including SPS, SLS, and SPS-plus...
June 1, 2016: JAMA Neurology
María Carmen Terencio, María Luisa Ferrándiz, María Carmen Carceller, Ramón Ruhí, Pere Dalmau, Josep Vergés, Eulàlia Montell, Anna Torrent, María José Alcaraz
CONTEXT: The efficacy of the combination chondroitin sulfate-glucosamine (CS-GlcN) in the treatment of knee osteoarthritis (OA) has been suggested in recent clinical studies. In vitro reports have also suggested anti-inflammatory and anti-resorptive effects of this combination. OBJECTIVE: The aim of this study was to characterize the effects of CS-GlcN on joint degradation in vivo including the assessment of inflammation and bone metabolism in a model of OA. MATERIALS AND METHODS: We have used the OA model induced by anterior cruciate ligament transection (ACLT) in ovariectomised rats...
April 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
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